Epidemiology, Natural History, and Outcomes of Primary Sclerosing Cholangitis: A Systematic Review of Population-based Studies

Trivedi, Palak; Bowlus, Christopher L.; Yimam, Kidist; Razavi, Homie; Estes, Chris

doi:10.1016/j.cgh.2021.08.039

Cited by 54 publications

(38 citation statements)

References 43 publications

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“…This also matched the estimated median transplant-free survival in transplant cohorts described by Boonstra et al [ 20 ], of which the study also highlighted the greatly improved median transplant-free survival in the population-based cohort of 21.3 years in comparison to 13.2 years in the transplant center cohort. Increased age at PSC diagnosis, development of cirrhosis and CCA has also been found to be significant predictors of transplant-free survival in keeping with the current literature [ 21 , 23 , 27 ].…”

Section: Discussionsupporting

confidence: 88%

Epidemiology and outcomes of primary sclerosing cholangitis: an Australian multicentre retrospective cohort study

Tan

Ngu²,

Worland³

et al. 2022

Hepatol Int

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Background and aims Little is known regarding the epidemiology and outcomes of patients with primary sclerosing cholangitis (PSC) in Australia. We, therefore, evaluated the epidemiology and clinical outcomes of PSC in a large cohort of Australian patients and compared these to the general population. Methods We conducted a multicentre, retrospective cohort study of PSC patients at nine tertiary liver centers across three Australian states, including two liver transplant centers. Results A total of 413 PSC patients with 3,285 person-years of follow-up were included. Three hundred and seventy-one (90%) patients had large duct PSC and 294 (71%) had associated inflammatory bowel disease. A total of 168 (41%) patients developed cirrhosis (including 34 at the time of PSC diagnosis) after a median of 15.8 (95% CI 12.4, NA) years. The composite endpoint of death or liver transplantation occurred in 49 (12%) and 78 (19%) patients, respectively, with a median transplant-free survival of 13.4 (95% CI 12.2–15) years. Compared to the general population, PSC accounted for a 240-fold increased risk of development of cholangiocarcinoma (CCA) and CCA-related death. CCA risk was increased with older age of PSC diagnosis, presence of dominant stricture and colectomy. Compared to same-aged counterparts in the general population, PSC patients who were diagnosed at an older age or with longer disease duration had reduced relative survival. Conclusion In this large retrospective cohort study of PSC patients in Australia, increased age and time from diagnosis was associated with increased mortality and morbidity particularly from CCA and development of cirrhosis, necessitating need for liver transplant.

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Section: Discussionsupporting

confidence: 88%

Epidemiology and outcomes of primary sclerosing cholangitis: an Australian multicentre retrospective cohort study

Tan

Ngu²,

Worland³

et al. 2022

Hepatol Int

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“…10 The estimated prevalence of IBD in patients with PSC varies greatly, ranging from 20% to 88%. [11][12][13] However, there was no meta-analysis performed to estimate the pooled prevalence rates. The prevalence of IBD in PSC patients in different geographical regions and the association between type of IBD and PSC subtypes and sex remains uncertain.…”

Section: Introductionmentioning

confidence: 99%

“…The association between PSC and IBD has been widely studied, 1 the pooled prevalence of PSC in IBD was 2.2% and was highest in South America and lowest in Southeast Asia 10 . The estimated prevalence of IBD in patients with PSC varies greatly, ranging from 20% to 88% 11–13 . However, there was no meta‐analysis performed to estimate the pooled prevalence rates.…”

Section: Introductionmentioning

confidence: 99%

Prevalence of inflammatory bowel disease in patients with primary sclerosing cholangitis: A systematic review and meta‐analysis

Zhang

Gao²,

et al. 2022

Liver International

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Background and Aim: Previous studies have established an association between primary sclerosing cholangitis (PSC) and inflammatory bowel disease (IBD), including Crohn's disease and ulcerative colitis (UC). The disease burden of IBD in PSC patients was not well estimated. The study aimed to quantify the pooled prevalence of IBD in PSC and to investigate whether subtypes of PSC and sex influence the prevalence of IBD.Methods: PubMed, Embase, and Web of Science were searched through November 2021 for studies reporting data on IBD among PSC patients. The outcomes were the prevalence of IBD in patients with PSC, as well as the association (odds ratio [OR]) of IBD in PSC according to subtype and sex. Results:Based on the analysis of 25 studies, the prevalence of IBD in patients with PSC was 71.1% (95% CI 68.2-75.1%), most commonly in UC (55.9%, 95% CI 52.5-59.3%). The pooled prevalence of IBD was 76.9% in Australia (95% CI 71.2-82.6%, 1 study), 75.9% (95% CI 69.5-82.3%, 4 studies) in North America, 70.9% (95% CI 65.8-76.0%, 17 studies) in Europe and 67.0% (95% CI 57.9-76.0%, 2 studies) in Asia. Male PSC patients had a higher prevalence of IBD (OR 1.67, 95% CI 1.52-1.83) and UC (OR 2.02, 95% CI 1.56-2.63) and a lower prevalence of CD (OR 0.77, 95% CI 0.67-0.88) than female patients. Large duct PSC patients had a higher prevalence of IBD (OR 2.57, 95% CI 2.03-3.25) and UC (OR 4.51,) than small duct PSC patients.

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“…PSC is one of the leading indications for liver transplantation in many countries. [1,7] Post-liver transplant course in PSC is further complicated by high rate of primary disease recurrence and graft loss as compared to other indications for liver transplantation. [8].…”

Section: Discussionmentioning

confidence: 99%

“…Liver transplant is the only definitive treatment in patients with advanced disease who develop decompensated cirrhosis. [1] There are limited data on outcomes of living donor liver transplant (LDLT) in PSC especially from India. Recurrence of PSC after liver transplant is of concern which can lead to graft failure and may require retransplant.…”

Section: Introductionmentioning

confidence: 99%

Good outcomes of living donor liver transplant in primary sclerosing cholangitis: an experience from North India

et al. 2022

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Background Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease. In the absence of effective medical therapy, liver transplant is the definitive treatment for advanced stage. However, recurrence of PSC after liver transplant is of concern which can lead to graft failure and may require retransplant. There are limited data on outcomes of living donor liver transplant (LDLT) in PSC. Also, in LDLT as donors are genetically related there can be an increased risk of recurrence. We conducted this retrospective study to analyze the outcomes of LDLT in PSC at a tertiary liver transplant center in north India. Methods We conducted a retrospective analysis of 3213 transplant recipients who underwent LDLT from January 2006 to May 2021. Of these 26 (0.80%) patients had PSC as indication for liver transplantation (PSC = 24, PSC-AIH overlap = 2). Data analysis was done to look for baseline demographics, clinical details, transplant outcomes, PSC recurrence, and survival. Results Mean age of study group was 42 (± 13.8) years and 19 patients (73.1%) were males. All patients had decompensated cirrhosis at the time of transplant. Mean CTP score and MELD score were 9.5 (± 1.8) and 18.9 (± 7.1), respectively. Sixteen patients received modified right lobe graft, seven extended right lobe graft and five patients received left lateral graft. Median graft weight and mean graft to recipient weight ratio (GRWR) were 633.5 ) grams and 1.23 (± 0.42), respectively. Most common biliary anastomosis was hepaticojejunostomy, done in 19 (73.1%) while duct to duct anastomosis was performed in 7 (26.9%) patients. Median follow-up was 96 (36-123) months. One patient had ulcerative colitis and none had cholangiocarcinoma. Two (7.7%) patients had bile leak during early post-transplant period. Three (11.1%) patients developed graft rejection and were managed successfully with steroid pulses. Three patients died during early post-transplant period while seven deaths occurred during long-term follow-up including one death due to COVID-19. Five (21.73%) patients had recurrence of PSC of which two patients had graft loss including one after retransplantation. The one year graft and patient survival rate was 88.5%. Conclusion LDLT can be performed in PSC with good long-term outcomes with a risk of PSC recurrence in about one-fifth patients.

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Epidemiology, Natural History, and Outcomes of Primary Sclerosing Cholangitis: A Systematic Review of Population-based Studies

Cited by 54 publications

References 43 publications

Epidemiology and outcomes of primary sclerosing cholangitis: an Australian multicentre retrospective cohort study

Epidemiology and outcomes of primary sclerosing cholangitis: an Australian multicentre retrospective cohort study

Prevalence of inflammatory bowel disease in patients with primary sclerosing cholangitis: A systematic review and meta‐analysis

Good outcomes of living donor liver transplant in primary sclerosing cholangitis: an experience from North India

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